Ivacaftor Brand Name– KALYDECO
What is Ivacaftor
Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator indicated for the treatment of cystic fibrosis (CF) in patients 12 months of age and older who have one mutation in the CFTR gene that is responsive to ivacaftor.
Ion channel modulators, such as ivacaftor, aim to correct the underlying cellular defect (chloride ion transport) responsible for CF instead of the severity of symptoms. Of note, ivacaftor monotherapy is not effective in patients who are homozygous for the F508del mutation, the most frequent genotype in patients with CF; approximately half of CF patients are homozygous for this allele.
However, in patients with an indicated mutation, ivacaftor therapy has been associated with improved forced expiratory volume in 1 second (FEV1), decreased risk of pulmonary exacerbation, increased quality of life, improved nutrition, and a large correction of elevated sweat chloride concentrations.
The Cystic Fibrosis Pulmonary Guidelines strongly recommend chronic use of ivacaftor to improve lung function and quality of life and reduce exacerbations in patients with a qualifying mutation.
In clinical trials of CF patients with at least one G551D-CFTR mutation (n = 213), mean improvement in FEV1 ranged from 10.6% to 12.5% in patients treated with ivacaftor (p less than 0.001).
Effects on pulmonary function were evident by 2 weeks, and significant effects were maintained throughout the 48-week trial period. The change in sweat chloride, a measure of CFTR activity, with ivacaftor ranged from -48 to -54 mmol/L (p less than 0.001).
The risk of pulmonary exacerbations was 55% lower in those treated ivacaftor compared to placebo in patients 12 years and older (n = 161).
Serious adverse reactions related to elevated liver transaminases and, in pediatric patients, the development of cataracts have been associated with ivacaftor. Hence, liver function test monitoring and eye exams are required at baseline and periodically throughout therapy
Indications
- cystic fibrosis
For the treatment of cystic fibrosis (CF) in patients who have one mutation in the CFTR gene that is responsive to ivacaftor
Side Effects
- abdominal pain
- acne vulgaris
- arthralgia
- cataracts
- diarrhea
- dizziness
- elevated hepatic enzymes
- headache
- hyperglycemia
- hypoglycemia
- infection
- musculoskeletal pain
- myalgia
- nasal congestion
- nausea
- pharyngitis
- rash
- rhinitis
- wheezing
Monitoring Parameters
- LFTs
- ophthalmologic exam
Contraindications
- breast-feeding
- cataracts
- hepatic disease
- infants
- pregnancy
- renal disease
- renal failure
- renal impairment
Interactions
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